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皮疹与间质性肺炎可能是致命组合:一例罕见的抗黑色素瘤分化相关基因5(MDA5)相关间质性肺病病例

Skin Rash and Interstitial Pneumonia Can Be a Fatal Combination: A Rare Case of Anti-Melanoma Differentiation-Associated Gene 5 (MDA5)-Associated Interstitial Lung Disease.

作者信息

Borio Giorgia, Terracciano Chiara, Buttafava Federico, Vercelli Andrea, Pagani Laura, Zanzani Chiara, Manicardi Alessandra, Magnacavallo Andrea, Poggiali Erika

机构信息

Emergency Department, Guglielmo da Saliceto Hospital, Piacenza, Italy.

Neurology Unit, Guglielmo da Saliceto Hospital, Piacenza, Italy.

出版信息

Eur J Case Rep Intern Med. 2021 Oct 18;8(10):002860. doi: 10.12890/2021_002860. eCollection 2021.

Abstract

UNLABELLED

We report the case of a 62-year-old male patient fully vaccinated for COVID-19, admitted to our emergency room for persistent fever associated with exertional dyspnoea, skin lesions, diffuse myalgias and arthralgias not responsive to broad-spectrum antibiotic and antiviral therapy, who developed a rapidly progressive refractory to treatment interstitial lung disease due to anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, that required mechanical ventilation and ECMO. Here, we highlight the importance of always considering alternative diagnoses, i.e. viral and autoimmune diseases, including anti-MDA5 antibody screening, when dealing with patients with a skin rash, seronegative polyarthralgias and interstitial pneumonia, or acute respiratory distress syndrome of unknown origin.

LEARNING POINTS

MDA5-associated dermatomyositis is a rare systemic syndrome associated with rapidly progressive and treatment-refractory interstitial lung disease.The anti-MDA5 antibody is the key biomarker for the diagnosis.Early diagnosis is crucial to promptly start aggressive immunosuppressive therapy with the aims of improving prognosis and reducing mortality.

摘要

未标注

我们报告了一例62岁男性患者,其已完成新冠病毒疫苗全程接种,因持续发热伴劳力性呼吸困难、皮肤病变、弥漫性肌痛和关节痛入院至我们的急诊室,这些症状对广谱抗生素和抗病毒治疗无反应,该患者因抗黑色素瘤分化相关基因5(MDA5)抗体导致快速进展且治疗难治的间质性肺病,需要机械通气和体外膜肺氧合(ECMO)。在此,我们强调在处理有皮疹、血清阴性多关节痛和间质性肺炎或不明原因急性呼吸窘迫综合征的患者时,始终考虑其他诊断,即病毒和自身免疫性疾病,包括抗MDA5抗体筛查的重要性。

学习要点

MDA5相关性皮肌炎是一种罕见的系统性综合征,与快速进展且治疗难治的间质性肺病相关。抗MDA5抗体是诊断的关键生物标志物。早期诊断对于及时开始积极的免疫抑制治疗至关重要,目的是改善预后并降低死亡率。

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Anti-MDA5-associated dermatomyositis.抗 MDA5 相关性皮肌炎。
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